Ewing’s sarcoma occurs in the bone or close to the bone, most often in adolescents between the age of 10 and 20. It occurs in about one teenager in 50,000 and accounts for about 30 percent of the bone cancers in children. This cancer most often is found in the arms and legs, particularly the thigh bone. It also may involve muscle and soft tissues surrounding the tumor. Ewing’s sarcoma cells can spread or metastasize to other areas of the body including bone marrow and the lungs.

Prior to adolescence, the number of boys and girls affected by this condition is equal. After adolescence, however, the number of men with the disease is slightly higher than women. This increase may be due to the increased rate of growth among males during adolescence.The following are the most common symptoms of Ewing’s sarcoma. Each child, however, may experience symptoms differently.

  • Pain around the site of the tumor
  • Swelling and redness around the site of the tumor
  • Fever
  • Weight loss and decreased appetite
  • Fatigue
  • Paralysis and incontinence if the tumor is in the spinal region
  • Symptoms related to nerve compression from a tumor such as numbness, tingling and paralysis

Treatment

A treatment program for Ewing’s sarcoma may include several approaches including surgery, radiation and chemotherapy.

  • Surgery: Surgery is used to remove a tumor or remove any tumor left after chemotherapy, which typically lasts about nine weeks. Surgery is performed if complete removal of the tumor is possible without damage to vital tissue or organs.
  • Radiation therapy: Radiation therapy is a painless procedure similar to X-rays. During therapy, a machine aims beams of high-energy X-rays at the cancer site to kill tumor cells. Some normal cells are damaged as well, but healthy cells have a greater ability to repair damage than do tumor cells. The goal is to damage normal cells as little as possible, while injuring tumor cells so they die or are unable to repair or reproduce themselves. Radiation therapy is used in combination with chemotherapy and sometimes surgery. Radiation for these tumors usually is external radiation from outside the body. Studies are evaluating the effectiveness of radiation implanted in the body during surgery.
  • Chemotherapy: Chemotherapy uses drugs to kill cancer cells. These drugs may be taken by pill, or injected in a vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the blood stream, travels through the body and can kill cancer cells throughout the body. When more than one drug is used, the treatment is called combination chemotherapy. For treating the Ewing’s family of tumors, surgery or radiation often is used to remove the local tumor and chemotherapy is used to kill any cancer cells that remain in the body.
  • Myeloablative Therapy: A supplement to the treatment options listed above is myeloablative therapy with stem cell support. This usually is reserved for patients who have resistant disease, recurrence of disease or widely disseminated disease. Myeloablative therapy is a very intense regimen of chemotherapy to destroy all cells that divide rapidly. These cells include some blood cells and hair cells, as well as cancer cells. Stem cells are self-renewing cells that create all of the other various types of blood cells. Stem cell support involves enriching the stem cells to increase the number of these important cells circulating in the blood after the chemotherapy has been given to kill the remaining tumor cells.

Treatment for the Ewing’s tumors depends on where the cancer is located, how far the cancer has spread, the stage of the disease, and the age and general health of the patient.

Localized Tumors

Treatment for localized Ewing’s tumors may be one of the following:

  • Combination chemotherapy followed by surgery
  • Radiation therapy or surgery in combination with radiation therapy
  • A clinical trial of intensified chemotherapy
  • Chemotherapy after surgery with or without stem cell transplant

Metastatic Tumors

Treatment for metastatic Ewing’s tumors may be one of the following:

  • Combination chemotherapy followed by radiation therapy or surgery
  • High-dose chemotherapy with or without radiation therapy plus additional stem cell support.
  • A clinical trial of intensive chemotherapy with multiple chemotherapy drug combinations

Recurrent Tumors

For recurrent tumors, treatment depends on where the cancer has recurred, how the cancer was treated before and the specific medical condition of each child. Chemotherapy may be used for children who did not previously receive chemotherapy. Radiation treatment may be given to reduce symptoms. Surgery may be used to remove tumors that have spread to the lungs or other organs. Clinical trials are testing new treatments.